Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging

Internal Medicine
Mizuho NaraNaoto Takahashi

Abstract

Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.

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Citations

Jun 23, 2020·Pediatric Blood & Cancer·Shogo MinamikawaRyojiro Tanaka
Sep 24, 2020·Annals of Hematology·Mitsuhiro AkiyamaTsutomu Takeuchi
Oct 23, 2020·Modern Rheumatology Case Reports·Yuta YamaguchiAtsushi Kumanogoh
Jun 8, 2021·Journal of Clinical and Experimental Hematopathology : JCEH·Tomoya OkamotoHideo Yagi

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Methods Mentioned

BETA
electrophoresis
biopsy

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Castleman disease is a rare disorder that involves an overgrowth of cells in the lymph nodes. Unicentric Castleman disease affects one lymph node, usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes, commonly located in the neck, collarbone, underarm and groin areas. Discover the latest research on Castleman disease here.